Articles with "otoferlin" as a keyword



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Otoferlin Is Required for Proper Synapse Maturation and for Maintenance of Inner and Outer Hair Cells in Mouse Models for DFNB9

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Published in 2021 at "Frontiers in Cellular Neuroscience"

DOI: 10.3389/fncel.2021.677543

Abstract: Deficiency of otoferlin causes profound prelingual deafness in humans and animal models. Here, we closely analyzed developmental deficits and degenerative mechanisms in Otof knock-out (Otof–/–) mice over the course of 48 weeks. We found otoferlin… read more here.

Keywords: required proper; outer hair; proper synapse; otoferlin required ... See more keywords

Activity-Dependent Phosphorylation by CaMKIIδ Alters the Ca2+ Affinity of the Multi-C2-Domain Protein Otoferlin

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Published in 2017 at "Frontiers in Synaptic Neuroscience"

DOI: 10.3389/fnsyn.2017.00013

Abstract: Otoferlin is essential for fast Ca2+-triggered transmitter release from auditory inner hair cells (IHCs), playing key roles in synaptic vesicle release, replenishment and retrieval. Dysfunction of otoferlin results in profound prelingual deafness. Despite its crucial… read more here.

Keywords: phosphorylation; otoferlin; camkii; ca2 affinity ... See more keywords